Idiopathic pulmonary fibrosis (IPF) is a lung disease in which lung tissue becomes progressively scarred and stiff. This makes it increasingly difficult to breathe.

There’s currently no cure for IPF, but new drugs have been able to slow the rate of decline and improve quality of life. Other treatment possibilities include supplemental oxygen, pulmonary rehabilitation to help you breathe better, and lung transplants. Experimental research studies are ongoing to find new treatments.

Early IPF treatment is important because it can slow the disease progression and improve your quality of life. It also contributes to knowledge of IPF and the results of different treatment courses on life expectancy. Treatments include:

  • Medication. New drug treatments may slow the rate of IPF lung scarring. This is important because lung scarring is irreversible. Medication can also reduce disease progression and possibly prevent mortality due to disease progression. While there are side effects to medication, treatment of IPF is crucial to future health.
  • Supplemental oxygen and physical therapy. These improve lung function, which can help you manage IPF and function more normally.
  • Exercise. Maintaining and increasing your muscle mass can improve your outlook, according to a 2016 study. A recent study shows that people with IPF who did pulmonary rehabilitation, a type of rehab program, showed significant improvement in function. The study also showed that there was a correlation with mortality if people cannot tolerate this rehab.
  • A lung transplant. This may significantly improve your outlook. The younger you are, the better qualified you’ll be for a transplant.

Your doctor will discuss with you what treatment options may be best for your particular case.

New drugs

The most important development in treating IPF is the availability of new drugs.

In 2014, the Food and Drug Administration (FDA) approved the use of two new drugs for IPF: nintedanib (Ofev) and pirfenidone (Esbriet). The drugs do not cure IPF, but they help to prevent further scarring and slow the progression of the disease.

Medical studies report that both drugs produced “a statistically significant slowing” in the decline of lung function. The same studies indicated that nintedanib produced somewhat better results than pirfenidone.

Supportive treatments

The standard care for IPF is supportive. A small portable oxygen tank can supply additional oxygen to help you breathe, especially when you’re more active. This is important for your comfort and also to prevent right-sided heart problems caused by low oxygen levels in your blood.

Pulmonary rehabilitation is a program designed to help you cope with IPF and improve your quality of life.

It involves exercises in:

  • breathing
  • stress reduction
  • education

One 2014 study showed that exercise training improved lung function.

Lung transplant

Lung transplantation can improve your quality of life and your lifespan, but it also has some risks. Experiences and outcomes of lung transplants vary. Keep in mind that lung transplants are only available to people who have severe and progressive lung dysfunction.

A transplant is also not a very easy option as the workup, surgery, and post-transplant care carry significant risks and involve lifelong monitoring afterward. While it can be a lifesaving option for some people, it’s definitely not an easy process.

Besides medical treatment options, there are things you can do to help manage and live better with the disease:

  • If you smoke, consider quitting. Smoking is connected with the occurrence of IPF, and smoking
    aggravates the disease.
  • If you’re overweight, losing weight to reach a moderate weight can be helpful. Having more weight can make it harder to breathe.
  • Keep up to date with flu and pneumonia vaccinations. Both illnesses can have a negative effect on the health of
    people with IPF.
  • If you have gastroesophageal reflux or sleep apnea, work with a healthcare professional to get treatment. These conditions can often be present in IPF patients.
  • Monitor your oxygen levels at home.
  • Consider joining an IPF support group to connect with others who are on the same medical journey and are likely having similar experiences.

If you have IPF, or you believe you may have IPF, please speak with a doctor regarding your options. You can also ask your doctor if pursuing some tests, such as a sleep study, could be helpful or necessary. Your doctor can help refer you to a specialist for IPF.